What is Dementia in Pick’s disease?
Dementia is a chronic or progressive brain disorder in which higher cortical functions are impaired, including memory, thinking, orientation, understanding, counting, learning ability, language and judgment. Consciousness is not darkened. Symptoms are present for at least 6 months, although a more progressive onset of dementia is possible. Long-term medication, vitamin B12 and B6 deficiencies, metabolic imbalances due to kidney and liver diseases, as well as endocrine pathology, vascular pathology, depression, infections, head injuries, alcoholism, and tumors contribute to dementia. In the diagnosis of dementia, additional symptoms, such as delirium, hallucinations, and depression, are also noted.
Causes of Dementia in Pick’s Disease
The disease is associated with atrophic processes in the frontal regions, often the dominant hemisphere, although a pair of frontal atrophy is possible. The disease is more often transmitted according to the dominant type, although recessive forms of transmission are also described. Atrophy is due to gliosis (argyrophil balls), although amyloid deposits are likely, resembling Glenner bodies. Atrophy leads to an increase in the anterior horns of the ventricles of the brain, compensatory hydrocephalus. Atrophy is genetically caused and transmitted by the recessive type. Theories for the development of Pick’s disease are similar to those of Alzheimer’s disease, although less developed. There is evidence that an excess of zinc is noted in the atrophy area.
Prevalence
Patients over the age of 60 years are affected, although some inadequate actions under the influence of external hazards, such as alcohol, at an earlier age can be attributed to provoking the disease’s pre-symptoms.
Symptoms of Dementia in Pick’s Disease
The disease proceeds in three stages, the initial symptoms have not been studied. Duration of illness up to 10 years.
In the first stage, symptoms of impaired social functioning and symptoms of unmotivated behavior are noted. As they grow, a pronounced frontal syndrome is noticeable. These include: sharpening of the egoistic orientation of the individual with disinhibition of instincts that are not controlled. The desire for the immediate implementation of instinct leads to the fact that individual actions seem to be facilitated and not motivated by the previous orientation of the individual and not relevant to the social environment. Patients can become sexually disinhibited, satisfy physiological needs without taking into account time and place. The importance of rigid stereotypes of behavior and peculiar conservatism increases. In speech, a symptom of a phonograph record, in which patients tell the same secrets, anecdotes, stories. Emotional life is characterized by unproductive euphoria (moria) or apathy. When the frontal symptoms increase, apathy or moria are no longer accompanied by periods, but by constant instinctive disinhibition, the so-called spontaneous “field” behavior. There are no amnestic disorders, the patients explain their misbehavior with ease and motivate with “incontinence” or impatience. In some cases, the disorder in peak dementia in the first stage is reminiscent of hebephrenic. When frontal basal atrophy is dominated by emotional and personality disorders, disinhibition and rigidity follow each other. With right hemisphere localization, anosognosia of abnormal behavior is combined with euphoria and complacency, with left hemispheric localization with depression. In convexital frontal atrophy, abnormal behavior is combined with apathy and abulia.
In the second stage, focal symptoms in the form of amnesia, aphasia, apraxia, agnosia, acalculia are noted, and at this stage it is difficult to distinguish dementia from Alzheimer’s disease, although epileptic seizures do not occur, echoraxia and echolalia are characteristic. A peculiar symptom is the hyperalgesia of the skin.
At the third stage, marasmus is observed with the transition to a vegetative coma with reduced muscle tone.
Diagnosis of Dementia in Pick’s Disease
Features of diagnosis lies in the fact that before the stages of cognitive deficit, especially in the field of memory, it is necessary to identify the defect of social functioning and the syndrome of inappropriate actions. On the EEG, a decrease in the voltage from the frontal leads can be seen, on CT, signs of frontal atrophy can be seen: expansion of the anterior horns of the lateral ventricles, thinning of the furrows and expansion of the subarachnoid spaces, density of the medulla in the sections of the atrophy are often reduced.
Differential diagnostics
It should be differentiated from Alzheimer’s disease, tumor and other processes in the frontal areas, hebephrenic schizophrenia.
Alzheimer’s disease begins with amnestic disorders, which are preceded by initial disorders of the usually affective and neurotic circle, are characterized by epileptic seizures and psychosis, and Pick’s disease – from disturbances in social functioning and the symptom of unmotivated actions at a later age (symptoms of E. Robertson).
With frontal tumors, local symptoms (frontal behavior, moria) are combined with cerebral symptoms (dizziness, headaches, nausea, characteristic changes in the fundus).
Similar disorders are observed with arteriovenous malformations in the system of the anterior cerebral artery, but in this case both cerebral and local frontal symptoms proceed paroxysmally.
Hebephrenic behavior, which may resemble a frontal lobe, is still observed in adolescence and middle age. Not verified cases of hebephrenia with onset at a later age.
Dementia Treatment for Pick Disease
Similar to Alzheimer’s. The syndrome of unmotivated actions deserves special attention of forensic psychiatrists in connection with possible asocial and unregulated behavior at a later age, in particular, when concluding contracts of purchase and sale, the appointment of guardianship.